Retinoblastoma classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Overview

Retinoblastoma may be classified into several subgroups based on the International Retinoblastoma Staging System (IRSS).[1] [2][3]

Classification

The International Classification for Intraocular Retinoblastoma, based upon the Philadelphia and Murphree systems is currently the classification being used by the Children's Oncology Group in multicenter treatment protocols.[1][2][3][4] The goal of International Classification of Retinoblastoma (ICRB) is to reflect the likelihood of ocular survival based on modern treatment techniques such as chemotherapy and focal therapy. The International Classification of Retinoblastoma (ICRB) was developed to better predict those with intraocular retinoblastoma who are likely to be cured without the need for external-beam radiation treatment or enucleation. International retinoblastoma classification predicts the overall survival in patients with retinoblastoma.[5]

The International Classification of Retinoblastoma staging system [4][6][7]
Groups Features
Group A
  • Small intraretinal tumors away from foveola and optic nerve
    • 3mm or smaller in the greatest dimension, confined to retina
    • Located further than 3 mm from the foveola and 1.5 mm from the optic disc.
Group B
  • Tumors confined to the retina.
    • Not in the group A
    • Tumor-associated subretinal fluid less than 3 mm from the tumor with no subretinal seeding.
Group C
  • Local disease with minimal subretinal or vitreous seeding with following caracteristics:
    • Discrete
    • Subretinal fluid, present or past, without seeding involving up to one-fourth of the retina
    • Local fine vitreous seeding may be present close to the discrete tumor
    • Local subretinal seeding less than 3 mm (2 DD) from the tumor
Group D
  • Diffuse tumor with significant vitreous or subretinal seeding
    • May be massive or diffuse
    • Subretinal fluid present or past without seeding, involving up to total retinal detachment
    • The diffuse or massive vitreous disease may include “greasy” seeds or avascular tumor masses
    • Diffuse subretinal seeding may include subretinal plaques or tumor nodules
Group E
  • Presence of any one or more of the following poor prognosis features
    • Tumor touching the lens
    • Tumor anterior to anterior vitreous face involving the ciliary body or anterior segment
    • Diffuse infiltrating retinoblastoma
    • Neovascular glaucoma
    • Opaque media from hemorrhage
    • Tumor necrosis with aseptic orbital cellulitis
    • Phthisis bulbi

References

  1. 1.0 1.1 Pratt CB, Fontanesi J, Lu X, Parham DM, Elfervig J, Meyer D (1997). "Proposal for a New Staging Scheme for Intraocular and Extraocular Retinoblastoma Based on an Analysis of 103 Globes". Oncologist. 2 (1): 1–5. PMID 10388023.
  2. 2.0 2.1 Shields CL, Mashayekhi A, Demirci H, Meadows AT, Shields JA (2004). "Practical approach to management of retinoblastoma". Arch Ophthalmol. 122 (5): 729–35. doi:10.1001/archopht.122.5.729. PMID 15136321.
  3. 3.0 3.1 Linn Murphree A (2005). "Intraocular retinoblastoma: the case for a new group classification". Ophthalmol Clin North Am. 18 (1): 41–53, viii. doi:10.1016/j.ohc.2004.11.003. PMID 15763190.
  4. 4.0 4.1 Shields CL, Mashayekhi A, Au AK, Czyz C, Leahey A, Meadows AT; et al. (2006). "The International Classification of Retinoblastoma predicts chemoreduction success". Ophthalmology. 113 (12): 2276–80. doi:10.1016/j.ophtha.2006.06.018. PMID 16996605.
  5. Retinoblastoma. Radiopedia(2015) http://radiopaedia.org/articles/retinoblastoma Accessed on October 10, 2015
  6. Zage PE, Reitman AJ, Seshadri R, Weinstein JL, Mets MB, Zeid JL, Greenwald MJ, Strauss LC, Goldman S (March 2008). "Outcomes of a two-drug chemotherapy regimen for intraocular retinoblastoma". Pediatr Blood Cancer. 50 (3): 567–72. doi:10.1002/pbc.21301. PMID 17729249.
  7. Novetsky DE, Abramson DH, Kim JW, Dunkel IJ (March 2009). "Published international classification of retinoblastoma (ICRB) definitions contain inconsistencies--an analysis of impact". Ophthalmic Genet. 30 (1): 40–4. doi:10.1080/13816810802452168. PMID 19172510.

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